Erythrokeratodermia variabilis. Report of three cases and review of the literature

Publisher Website
Google Scholar
Abstract
Three patients with erythrokeratodermia variabilis (EKV), representing 3 consecutive generations of the same family, az e presented and the literature is reviewed. The major features of this disorder ar onset during early infancy; autosomal dominant mode of inheritance; and distinctive cutaneous lesions which consist of sharply demarcated, hyperkeratotic plaques with a geographic outline and which frequently are associated with sharply outlined and independent areas of erythrodermia. The comparative clinical and hiatopathologic features of EKV and congenital ichthyosiform erythroderma are briefly reviewed. Most authors suggest that EKV is a localized variant of congenital Ichthyosiform erythroderma.

This publication has 1 reference indexed in Scilit: